Health Library

Usher Syndrome

What is Usher syndrome?

Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Retinitis pigmentosa is a progressive degeneration of the retina that causes night blindness and reduction of peripheral vision (side vision). Some people also have varying problems with balance.

Usher syndrome is passed from parents to their children genetically.

What are the different types of Usher syndrome?

There are three types of Usher syndrome:

US type 1 (US1)--characteristics include:

Profoundly deaf from birth

Do not usually benefit from hearing aids

Severe balance problems

Vision problems begin by age 10

Blindness eventually occurs

US type 2 (US2)--characteristics include:

Moderate to severe hearing problems

Usually benefit from hearing aids

Use speech to communicate

Normal balance

Retinitis pigmentosa begins in teenage years

US type 3 (US3)--characteristics include:

Born with normal hearing

Hearing problems develop in teenage years

Near normal balance

Deafness by late adulthood

Retinitis pigmentosa begins around puberty

Blindness by mid-adulthood

How is Usher syndrome diagnosed?

Special tests assist in the diagnosis of Usher syndrome, including:

Electronystagmography (ENG) to detect balance problems

Electroretinography (ERG) to detect retinitis pigmentosa

﻿Audiologic (hearing) evaluation and testing

Treatment for Usher syndrome

Specific treatment for Usher syndrome will be determined by your health care provider based on:

Your age, overall health, and medical history

Extent of the disease

Your tolerance for specific medications, procedures, or therapies

Expectations for the course of the disease

Your opinion or preference

At present, there is no known cure for Usher syndrome. The best treatment, however, is early identification so that education programs can begin as soon as possible. Treatment may include: